title: A strategy for glycine encephalopathy therapy.
abstract: An inherited defect in the glycine cleavage enzyme results in the condition of neonatal glycine encephalopathy which has not responded to the current innovative methods of therapy. A re-examination of the enzyme structure and metabolic pathways, leads us to recommend future clinical evaluation of (1) vitamin-responsiveness, e.g., pyridoxine, folate and lipoic acid, (2) methionine, (3) N5, N10-methylene tetrahydrofolate and (4) alpha-methylserine therapy during the critical period of neonatal brain growth and development.
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy*; D000594:Amino Acid Oxidoreductases / Q000378:metabolism; D050960:Aminomethyltransferase; D000818:Animals; D001928:Brain Diseases, Metabolic / Q000151:congenital* / Q000188:drug therapy; D002352:Carrier Proteins / Q000378:metabolism; D055598:Chemical Phenomena; D002621:Chemistry; D005998:Glycine / Q000378:metabolism*; D050841:Glycine Dehydrogenase (Decarboxylating); D006801:Humans; D019877:Hydroxymethyl and Formyl Transferases; D007223:Infant; D007231:Infant, Newborn; D008715:Methionine / Q000627:therapeutic use; D051379:Mice; D012694:Serine / Q000031:analogs & derivatives / Q000627:therapeutic use; D013763:Tetrahydrofolates / Q000627:therapeutic use; D014166:Transferases / Q000378:metabolism
chemical_list: D002352:Carrier Proteins; D013763:Tetrahydrofolates; C013123:5,10-methylenetetrahydrofolic acid; C035563:2-methylserine; D012694:Serine; D008715:Methionine; D000594:Amino Acid Oxidoreductases; D050841:Glycine Dehydrogenase (Decarboxylating); D014166:Transferases; D019877:Hydroxymethyl and Formyl Transferases; D050960:Aminomethyltransferase; D005998:Glycine